scholarly journals Management of Patient with Sturge-Weber Syndrome: a Case Report

2015 ◽  
Vol 9 (2) ◽  
pp. 102-104
Author(s):  
AKM Bazlul Karim ◽  
Mohammad Monirul Islam ◽  
Md Alamgir Hossain ◽  
Abu Md Shakurul Alam
Keyword(s):  
Case Report ◽  
Right Hemisphere ◽  
Resonance Imaging ◽  
Professional Counseling ◽  
Mentally Disabled ◽  
Weber Syndrome ◽  
The Face ◽  
Sturge Weber Syndrome ◽  
Slow Activity ◽  
The Right

Sturge-Weber syndrome sometimes referred to as encephalotrigeminal angiomatosis, is a rare congenital neurological and skin disorder. This is case report of 7 years old mentally disabled boy, with long-standing seizures, with a portwine nevi on the right side of the face along the distribution of trigeminal nerve. Interictal encephalogram showed bilateral slow activity, pronounced in the right hemisphere, with epileptogenic activity in the right fronto-parietal region. Computerized tomography and magnetic resonance imaging showed intracranial calcifications and atrophy of the right hemisphere of brain. Professional counseling and support in addition to drug treatment provide help to patients and their family to overcome their problems and improve the treatment outcome.Faridpur Med. Coll. J. 2014;9(2): 102-104

scholarly journals Sturge-Weber Syndrome

KYAMC Journal ◽  
2017 ◽  
Vol 4 (1) ◽  
pp. 366-368
Author(s):  
Md Alamgir Hossain ◽  
AKM Bazlul Karim ◽  
Abu Md Shakurul Alam
Keyword(s):  
Right Hemisphere ◽  
Resonance Imaging ◽  
Mentally Disabled ◽  
Weber Syndrome ◽  
The Face ◽  
Sturge Weber Syndrome ◽  
Slow Activity ◽  
The Right ◽  
Venous Angiomas ◽  
Professional Counselling

Introduction: Sturge-Weber syndrome sometimes referred to as encephalotrigeminal angiomatosis, vascular malformation with capillary venous angiomas involving face, choroid of eye, leptomeninges, is a rare congenital neurological and skin disorder.Case presentation: This is case report of 7 years old mentally disabled boy, with long-standing seizures, with a port-wine nevi on the right side of the face along the distribution of trigeminal nerve. Interictal encephalogram showed bilateral slow activity, pronounced in the right hemisphere, with epileptogenic activity in the right fronto-parietal region. Computerized tomography and magnetic resonance imaging showed intracranial calcifications, abnormally large veins in deep medullary and subependymal periventricular region, atrophy of the right hemisphere of brain and ipsilateral thickening of skull.Conclusion: Professional counselling and support in addition to drug treatment an provide help to patients and their family to overcome their problems and improve the treatment outcome.KYAMC Journal Vol. 4, No.-1, July 2013, Page 366-368

scholarly journals Bilateral Sturge Weber Syndrome- a rare case report

2013 ◽  
Vol 5 (1) ◽  
pp. 129-132 ◽  
Author(s):  
P Singh ◽  
S Singh
Keyword(s):  
Case Report ◽  
Rare Case ◽  
Unusual Case ◽  
The Other ◽  
Advanced Stage ◽  
Weber Syndrome ◽  
Rare Case Report ◽  
The Face ◽  
Bilateral Condition ◽  
Sturge Weber Syndrome

Background: Sturge-Weber syndrome is a rare congenital neuro- oculo- cutaneous disorder. Objective: To report a very rare unusual case of bilateral manifestation of Sturge Weber syndrome. Case: We report an unusual case of a 17-year-old female with advanced stage of bilateral glaucoma associated with facial nevus extending to the other half of the face as well and bilateral intracranial calcification. Conclusion: Sturge -Weber syndrome can manifest as a bilateral condition. Nepal J Ophthalmol 2013; 5(9):129-132 DOI: http://dx.doi.org/10.3126/nepjoph.v5i1.7841

scholarly journals Isolated leptomeningeal angiomatosis in the sixth decade of life, an adulthood variant of Sturge Weber Syndrome (Type III): role of advanced Magnetic Resonance Imaging and Digital Subtraction Angiography in diagnosis

BMC Neurology ◽  
2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Vetrivel Muralidharan ◽  
Gaetano Failla ◽  
Mario Travali ◽  
Tiziana Liliana Cavallaro ◽  
Marco Angelo Politi
Keyword(s):  
Clinical Presentation ◽  
Subcortical White Matter ◽  
Digital Subtraction ◽  
Resonance Imaging ◽  
Radiological Findings ◽  
Hyperintense Signal ◽  
Right Cerebral Hemisphere ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome ◽  
The Right

Abstract Background Sturge-Weber syndrome (SWS) is primarily diagnosed in pediatric population, but clinical presentation in late adulthood is rarely reported. Evolution of radiological findings in the adulthood variant of SWS with isolated leptomeningeal angiomatosis has never been reported to our knowledge. Case presentation We report here a case of an isolated temporo-parieto-occipital leptomeningeal angiomatosis on the right cerebral hemisphere in a sixty-two-year-old male who presented with generalized seizure, GCS score 14/15 (E4 V4 M6) with equal and reacting pupils, psychomotor slowing, left hemineglect and grade 4 power in the left upper and lower limbs. Over a period of 48 h his neurological status deteriorated, but recovered spontaneously over a week on titration with anticonvulsants. He had a prior history of treatment for focal leptomeningitis, three years ago. Cerebrospinal fluid (CSF) analysis showed glucose of 75 mg/dL, proteins of 65 mg/dL and culture grew no organisms. On follow-up, he had intermittent episodes of focal seizure for two years. Initial, computed tomography of brain showed hyperdense lesion in the parieto-occipital convexity subarachnoid space on the right cerebral hemisphere mimicking subarachnoid hemorrhage and computed tomography angiography showed no significant abnormality. Magnetic resonance imaging (MRI) of brain showed intense pial enhancement in the right temporo-parieto-occipital region with a subtle T2W hyperintense signal in the underlying subcortical white matter without edema or infarct or mass effect. Digital subtraction cerebral angiography (DSA) showed hypertrophy of the cerebral arteries, arteriolo-capillary bed and venules in the right temporo-parieto-occipital territory associated with early arterio-capillary and venous opacification. Serial MRI done after six months, one and two years showed increase in the T2W hyperintense signal in the subcortical white matter and cortical atrophy with no changes in the pial enhancement. MR perfusion imaging showed reduced cerebral blood flow (CBF) and cerebral blood volume (CBV) in the right parieto-temporo-occipital cortical and subcortical regions and increased perfusion in the leptomeninges with reduction of the NAA / Cr ratios in spectroscopy. Conclusion Conglomeration of various radiological findings in MRI, Perfusion, MRS and DSA with the clinical presentation can aid in establishing the diagnosis of this rare presentation of SWS-type 3 variant in late adulthood.

scholarly journals A case report of a pregnant woman with Sturge-Weber syndrome

2020 ◽  
Vol 48 (4) ◽  
pp. 030006052091370
Author(s):  
Ying Luo ◽  
Yang Yang ◽  
Xiu Chen
Keyword(s):  
Magnetic Resonance Imaging ◽  
Pregnant Woman ◽  
Cerebral Hemisphere ◽  
Surgical Intervention ◽  
Port Wine ◽  
Resonance Imaging ◽  
Intracranial Vessel ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome ◽  
The Right

A 23-year-old pregnant woman presented with headache and paroxysmal spasm of the right limb. She was born with a port-wine birthmark around the left eye frame. Magnetic resonance imaging revealed evidence of atrophy, calcification and vascular malformation in the left cerebral hemisphere. She was diagnosed with Sturge-Weber syndrome. She gave birth to a child without a port-wine birthmark through a caesarean section and her headache eased without surgical intervention of the intracranial vessel hyperplasia. This case suggests that pregnant women with SWS can deliver safely.

scholarly journals A Rare Case of Encephalotrigeminal Angiomatosis: A Case Report

2017 ◽  
Vol 7 (1) ◽  
pp. 13
Author(s):  
Lisa Elizabeth Jacob ◽  
Anuna Laila Mathew ◽  
Omal Pattali Mohanan ◽  
Thomas Abraham ◽  
Jerin Thomas ◽  
...  
Keyword(s):  
Case Report ◽  
Health Care Professionals ◽  
Rare Case ◽  
Dental Treatment ◽  
Disease Process ◽  
Adequate Knowledge ◽  
Weber Syndrome ◽  
Native Speakers Of English ◽  
Sturge Weber Syndrome ◽  
The Right

Aim: Sturge–Weber Syndrome, also known as encephalotrigeminal angiomatosis, is an uncommon, nonhereditary developmental anomaly. Methodology: A 48-year-old woman presented for routine dental treatment. She had a history of seizures and had a port wine stain on the right side of her face, which followed the distribution of the trigeminal nerve. Skull radiographs revealed “tram-track” calcifications. Conclusions: The early diagnosis of Sturge–Weber Syndrome requires a multidisciplinary approach. Oral health care professionals need adequate knowledge and understanding of the disease process to help diagnose and treat these patients.  How to cite this article: Jacob LE, Mathew AL, Omal PM, Abraham T, Thomas J, Varghese S. A Rare Case of Encephalotrigeminal Angiomatosis – A Case Report Int Dent Res 2017;7:13-6. Linguistic Revision: The English in this manuscript has been checked by at least two professional editors, both native speakers of English.

scholarly journals Type III roach scale Sturge-Weber syndrome associated with an extracranial lipoma: A case report

2019 ◽  
Vol 72 (1-2) ◽  
pp. 47-50
Author(s):  
Stefan Stojanoski ◽  
Dusko Kozic ◽  
Milos Lucic ◽  
Katarina Koprivsek
Keyword(s):  
Magnetic Resonance Imaging ◽  
Case Report ◽  
Magnetic Resonance ◽  
Imaging Modality ◽  
Correct Diagnosis ◽  
Psychomotor Development ◽  
Cortical Atrophy ◽  
Resonance Imaging ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome

Introduction. Sturge-Weber syndrome is a neurocutaneous disorder characterized by neurological features such as headaches, developmental delay, mental retardation and seizures, facial anomalies such as port-wine stain and glaucoma which is the most common ocular manifestation. The main neuroimaging findings in patients with Sturge-Weber syndrome are leptomeningeal angiomatosis and corticopial calcifications associated with underlying cortical atrophy. The purpose of this report is to present a rare case of a patient with seizures whose magnetic resonance imaging findings suggested a Sturge-Weber syndrome variant. Case Report. We report a case of a 14-yearold boy with a two year history of well controlled generalized tonic clonic seizures with visual aura, who was admitted to our institution for neuroimaging examination. Neuropsychological testing showed normal cognitive and psychomotor development. Electroencephalography revealed unilateral runs of right occipital spikes with secondary generalization. Neuroimaging findings showed focal cerebral leptomeningeal enhancement in the right parasagittal occipital region associated with focal cortical atrophy, whereas susceptibility weighted imaging showed hypointense intracortical calcification and hyperplastic enhanced ipsilateral choroid plexus. The computed tomography confirmed cortical calcifications. Also, an overlying parietooccipital subcutaneous lipoma was found in the innervation field of the ophthalmic nerve. Conclusion. Magnetic resonance imaging is the key imaging modality that confirmed the clinical suspicion of Sturge-Weber syndrome based on a physical and neurological examination. Neither magnetic resonance imaging nor clinical examination is sufficient for a correct diagnosis.

scholarly journals Provocation and prediction of visual peripersonal neglect-like symptoms in preoperative planning and during awake brain surgery

2021 ◽  
Author(s):  
Viktória Tamás ◽  
Gabriella Sebestyén ◽  
Szilvia Anett Nagy ◽  
Péter Zsolt Horváth ◽  
Ákos Mérei ◽  
...  
Keyword(s):  
Preoperative Planning ◽  
Right Hemisphere ◽  
Superior Temporal Gyrus ◽  
Low Grade ◽  
Navigated Transcranial Magnetic Stimulation ◽  
Resonance Imaging ◽  
Parietal Area ◽  
Routine Magnetic Resonance Imaging ◽  
Visuospatial Functions ◽  
The Right

AbstractNeglect is a severe neuropsychological/neurological deficit that usually develops due to lesions of the posterior inferior parietal area of the right hemisphere and is characterized by a lack of attention to the left side. Our case is a proven right-handed, 30-year-old female patient with a low-grade glioma, which was located in the temporo-opercular region and also in the superior temporal gyrus of the right hemisphere. Upon presurgical planning, the motor, language, and visuospatial functions were mapped. In order to achieve this, the protocol for routine magnetic resonance imaging and navigated transcranial magnetic stimulation has been expanded, accordingly.

71. Sturge–Weber syndrome + cortical dysplasia. A dual pathology case report

2016 ◽  
Vol 127 (9) ◽  
pp. e319
Author(s):  
Areli Rosario Suarez-Roman ◽  
Enoe Cruz-Martinez ◽  
Yokary Amor Mellado-Ortiz ◽  
Ernesto Ramirez-Navarrete ◽  
Paul Shkurovich-Bialik
Keyword(s):  
Case Report ◽  
Cortical Dysplasia ◽  
Dual Pathology ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome

scholarly journals Double trouble: Bilateral cerebral involvement in Sturge-Weber syndrome

2015 ◽  
Vol 19 (1) ◽  
Author(s):  
Narosha Adroos ◽  
Janet Smal ◽  
Farhana E. Suleman
Keyword(s):  
Mental Retardation ◽  
Intracranial Calcification ◽  
Clinical Correlation ◽  
Bilateral Disease ◽  
Intractable Seizures ◽  
Weber Syndrome ◽  
Cerebral Involvement ◽  
The Face ◽  
Sturge Weber Syndrome ◽  
Prognostic Implications

Sturge-Weber syndrome, also known as encephalotrigeminal angiomatosis or meningofacial angiomatosis, is characterised in its classical form by a congenital, usually unilateral, ‘portwine stain’ (capillary naevus) on the face, convulsions, typical intracranial calcification and some degree of mental retardation and hemiparesis. The clinical correlation of intractable seizures with the presence of bilateral intracranial disease has management and prognostic implications, thus making the presence of bilateral disease an important factor to all those involved in the management of the child with Sturge-Weber syndrome.

Encephalotrigeminal Angiomatosis: A Review with Emphasis on Head and Neck Manifestations

Neurographics ◽  
2021 ◽  
Vol 11 (3) ◽  
pp. 166-174
Author(s):  
B.R. Larsen ◽  
M.S. Rosztoczy ◽  
D.C. Van Tassel ◽  
C.R. Larson ◽  
S.A. Avedikian ◽  
...  
Keyword(s):  
Head And Neck ◽  
Soft Tissues ◽  
Clinical Findings ◽  
Imaging Features ◽  
Facial Soft Tissue ◽  
Vascular Abnormalities ◽  
Weber Syndrome ◽  
The Face ◽  
Sturge Weber Syndrome ◽  
Tissue Abnormalities

Encephalotrigeminal angiomatosis, also known as Sturge-Weber syndrome or Sturge-Weber-Dimitri disease, is a sporadic congenital neurocutaneous syndrome characterized by angiomas of the face, eyes, and meninges. The typical clinical findings include seizures, a port-wine facial nevus, hemiparesis, and developmental delay. The classic intracranial imaging features of Sturge-Weber syndrome include cerebral hemispheric atrophy, hypervascularity and/or calcification of the cortex, and ipsilateral choroid plexus enlargement. Extracranial findings primarily include the vascular abnormalities of the ocular and facial soft tissues as well as hypertrophy of the calvarial and maxillofacial osseous structures. Additionally, we report involvement of the extraocular muscles, salivary glands, and lacrimal glands, findings that have not been well described in the literature. While a developmental venous pathology underlying Sturge-Weber syndrome has been proposed resulting in many of the findings, the mechanism behind the facial soft-tissue abnormalities and the clinical sequelae associated with these glandular abnormalities is still uncertain.Learning Objective: To recognize typical clinical and various imaging manifestations of Sturge-Weber syndrome, including head and neck and intracranial imaging findings

Sign in / Sign up

Export Citation Format

Share Document